Neonates are obligate nasal breather until they are at least two to five months old. Congenital nasal airway obstruction is one of the commonest causes of respiratory problem in newborn. Congenital nasal pyriform aperture stenosis (CNPAS) was first described by Brown et al in 1989 [1] and is a rare cause of nasal airway obstruction which may clinically mimic choanal atresia.(Copied from article)
Subcutaneous fat necrosis is a recognised complication of hypothermia. There appears to be a resurgence in this condition when therapeutic hypothermia emerges as a standard of care for asphyxiated infants. The TOBY trial reported an incidence of 0.1% for 1239 infants cooled.(Copied from article)
Hepatopulmonary syndrome (HPS) is characterized by the triad of liver disease, pulmonary vascular dilatation and arterial oxygenation abnormality. It occurs in 4% to 47% in patient with liver cirrhosis [1]. We describe 2 cases of HPS with recovery from hypoxaemia after liver transplantation.(Copied from article)
Child abuse's trend has been on the rise in Malaysia. Despite strong legislative rules of Child Act 2001, the response of the community is believed to be inadequate. Various factors have been highlighted to ensure effective management of the issue. Child abuse is considered a social emergency due to its complexity and involvement of emotional, family dynamic and domestic instability. The success of the managing these cases largely depends on our urgency and collaborative partnership between multidisciplinary team members, to improve on the welfare of these children, albeit a slower outcome. The community as a whole has to be proactive in reporting hidden cases to avoid mortality or morbidity, as a result of the perpetrator's actions.
Objectives: This study was conducted with two objectives, i) to assess the prevalence of malnutrition among children and adolescents with learning disability (LD) in Kelantan, a rural state located at the north-eastern region of Peninsular Malaysia; and ii) to examine the associations of participants’ socioeconomic and feeding characteristics with their body mass index (BMI).
Methods: A total of 271 children and adolescents with LD aged between 4 to 19 years old were recruited from 32 community-based rehabilitation centres using purposive sampling method. Standing height and body weight of participants were measured. Socioeconomic and feeding information were obtained from their primary caregivers through interviews using structured questionnaire. Multiple linear regression analyses were performed to examine the associations of socioeconomic and feeding characteristics with participants’ BMI.
Results: The prevalence of underweight among children and adolescents with LD was 22.5%; while another 22.1% of them were overweight and obese. Multiple linear regression models showed that being Down’s syndrome (Adjusted regression coefficient β=2.63, p
Objective: To describe the effect of mycophenolate mofetil in Pakistani children with steroid dependent and steroid resistant nephrotic syndrome. Methods: This is cross sectional retrospective review of 16 patients; 9 boys and 7 girls (11 SD/FRNS and 5 SRNS) for a period of 4.8 years. This study was conducted in Mayo hospital and Fatima Memorial hospital specialist care centre, Lahore involving urban and suburban population. Results: The median age of the group was 4 years (1.6 to 12.6 years). Seven patients had histological diagnosis of MCN, 3 had diffuse mesangial proliferation, one of membranoproliferative glomerulonephritis and 4 had FSGS. Out of 5 SRNS 4 were found to have FSGS and 1 had membranoproliferative glomerulonephritis (MPGN). A total of three patients were completely off steroids and in two patients MMF was also successfully stopped. Number of relapses /patient /year calculated by applying Wilcoxan signed rank test was found to be 4.31 + 0.87(3.00-6.00 /patient/year) before starting MMF, which dropped to 1.12 + 0.718 (0.00- 2.000 /patient/year) after starting MMF, p=0.0001. Reduction in steroid dose from mean of 0.85 + 0.18 mg/kg/day to 0.3mg/kg/day + 1.56 was achieved in 12 months, p
Intravenous immunoglobulin (IVIG) therapy in Kawasaki disease (KD) has been shown to reduce coronary artery aneurysm by 4-5%. However, we still observed significant number of coronary aneurysm post IVIG in our centre. The objectives of this study were to determine the prevalence of coronary artery abnormality (CAA) and the associated risk factors.
Designs: Retrospective descriptive study. Method: A retrospective study performed on children with KD from 1 st January 2005 to 30 th July 2010. Japanese Ministry of Health criteria were used to classify coronary arteries abnormality. Children with incomplete or atypical KD were excluded. Data were extracted from Pediatric Cardiology Clinical Information System.
Results: A total of 126 KD were diagnosed during the study period with 69% were male and 52.4% were Chinese. The median age of diagnosis was 1.4yr [Q1, 0.6yr Q3, 2.3yr]. Of these 126, 118 (93.7%) received IVIG within 10 days of illness. Ten patients (7.9%) required more than one dose of IVIG. CAA were noted in 28 (22.2%) patients with 21 ectasia, 4 small fusiform, one small saccular and 2 medium fusiform aneurysm. Of these 28, 22 who had IVIG within 10 days of illness (18 ectasia, 3 small and one medium coronary aneurysm) Significant risk factors for CAA were older children (2.3 v s 1.7yr, p=0.03), presentation after 10days of illness (p=0.006) and required more than 2gm/kg of IVIG (p=0.04).
Conclusion: CAA in complete KD treated with IVIG was 22.2% with 5.5% significant aneurysm. Risk factors for coronary abnormality were older children, late presentation and require more of IVIG of than 2gm/kg.
Objective: This study assessed the pattern of clinical course of hematological disorders in children diagnosed by bone marrow aspiration/biopsy in a tertiary care centre. Setting: The study was conducted at the Department of Pediatrics, Lahore General Hospital, Pakistan. Design: A retrospective descriptive study. Duration of study: Jan 2006 to Dec 2010. Methods: The clinical and laboratory data of 250 patients including complete history, physical examination, investigations and bone marrow examination reports were collected and then analyzed retrospectively. On the basis of these data, relative frequency of different hematological disorders was determined. Results: A total of 250 patients were selected during this study period where their bone marrow was sent for the investigations. Out of these cases, double deficiency anemia was the commonest diagnosis (22%) followed by aplastic anemia (13.6%), megaloblastic anemia (13.2%) and iron deficiency anemia (5.6%). For hematological malignancies, acute lymphoblastic leukemia (ALL) was observed in 27 cases (10.8%) followed by acute myeloid leukemia (AML) in 12 cases (4.8%), lymphoma in 8 cases (3.2%) and chronic myeloid leukemia (CML) in only two cases. Idiopathic thrombocytopenic purpura (ITP) was reported as frequent as 13.2% (33 cases). Conclusion: The pattern of non malignant hematological disorders in children diagnosed by bone marrow aspiration/biopsy was more common than malignant conditions. Double deficiency anemia was the commonest non malignant condition followed by aplastic anemia, idiopathic thrombocytopenic purpura and megaloblastic anemia. ALL was the most common presentation of the hematological malignancy.
Asthma is considered as heterogeneous multidimensional disorder due to variable phenotypic presentation. Phenotype is defined as a cluster of either clinical or pathologic features, which tends to be associated with the understanding the mechanisms of the disease. Asthma is typically characterized by airway inflammation, variable airway obstruction, bronchial hyper-reactivity, smooth muscle hypertrophy and apparent reversible airflow obstruction by bronchodilators. ‘Asthma syndrome’ is a term to describe complex pathophysiology of the condition which is not exclusive to allergen triggered episodes. Diagnosing childhood asthma is difficult, due to the similarity of symptoms and overlapping with other wheezing conditions. The precise mechanism for asthma exacerbation, for each individual phenotype is not fully understood. However, it is influenced by genetic interaction with variety of external environmental stimuli. The current understanding on asthma phenotypes were interpreted based on age of onset, associated triggers, clinical aspects, physiologic parameters and type of inflammation (Table 1). Due to the illdefined of the current phenotypic definition and disagreement among the respirologists, it is becoming a challenge to label specific phenotype with certainty. Accurate definition of each phenotype should therefore be helpful to provide better understanding of different mechanistic pathways and focusing on targeted therapy for individual phenotype. (Copied from article).
The report describes a pilot study of cognitive-behavioural social skills program conducted on primary school children in Kelantan, Malaysia. Designs: Uncontrolled pre- and post- single design study. Methods: Children aged 9 - 11 referred by school teachers for behavioural problems were put into Petersen’s Stop-Think-Do cognitive-behavioural social skills program for 6 sessions delivered by a clinical psychologist. Main outcome measure was Goodman’s Strengths and Difficulties Questionnaires; to assess child’s emotional and behavioural functioning rated by parents and children themselves. Results: The results showed positive short-term effects of the program at improving some aspects of children’s psychological functioning, however they are not significant. Findings are limited to several issues including subjects’ drop-out, problems in generalizing learnt behaviour, and the use of single outcome measure. Conclusion: Whilst the program could possibly improve children behavioural functioning more dramatically, the implementation of such program in school setting must be organized in full collaboration and assistance from the parents and school administrators.
Forty thousand children die each day in developing countries; which is approximately 28 dead children every minute. In many of these countries 25% of their children do not even live long enough to celebrate their fifth birthday. These mortality statistics represents a human tragedy affecting our very youngest. The United Nations Children's Fund (UNICEF) has identified seven simple, practical and cost-effective activities to help save the lives of our children. The acronym GOBI FFF - Child Survival Strategy describes these 7 measures. The B in the acronym represents beastfeeding which is a pivotal and fundamental component of this child survival strategy.
Generally, the karyotype profile of Down Syndrome has been reported to be full trisomy 21 in 92% of patients, mosaic trisomy 21 in 4% of patients and translocation involving chromosome 21 in 4% of patients in most of the population groups worldwide. But, karyotype analysis of 149 DS patients at the Human Genome Center, USM, during the past five years revealed that free trisomy accounted for 94.6%, mosaic trisomy 21 for 4.7% and translocation involving chromosome 21 in 0.7% of the Down Syndrome etiology in North East Malaysian population, indicating a low frequency of translocation DS in this region. Here, we report one case of translocation Down Syndrome encountered during karyotype analysis of 149 DS cases. Karyotype showed a robertsonian translocation where an entire extra chromosome 21 was attached to the centromere of one of the chromosome 14, resulting in a derivative chromosome 14 with attached chromosome 21. Karyotype analysis of the parents revealed a normal 46,XY pattern for father and 46,XX pattern for mother indicating that this robertsonian translocation had arisen de novo either prior to or at conception.
Endobronchial tuberculosis is an uncommon manifestation involving the tracheobronchial tree. The clinical presentation is typically non-specific. We report this unusual complication of pulmonary tuberculosis initially diagnosed as foreign body in a 16-month-old child.
Objectives: 1) To establish the incidence of rotavirus infection in paediatric patients admitted to a private hospital in Malaysia with a diagnosis of community-acquired acute gastroenteritis. 2) To determine whether patients with rotaviral acute gastroenteritis have greater disease severity. Design Retrospective cross-sectional study. Setting An urban private hospital in Malaysia. Participants All paediatric patients with a discharge diagnosis of acute gastroenteritis (AGE) admitted to the hospital between September 2005 and March 2006. A total of 261 patients were included in the study. Results Rotavirus infection contributed to 54% of paediatric hospital admissions with discharge diagnoses of acute gastroenteritis. 74% of children with rotavirus infection were aged 5 years and below. This study demonstrated that patients with rotaviral gastroenteritis had greater disease severity, as measured by two indicators. Firstly, the average length of hospital stay was longer in patients who were rotavirus positive (5.14 days) compared to those who were rotavirus negative (4.81 days). These results were statistically significant (p
Spinal Muscular Atrophy (SMA) is a heredity neuromuscular disorder and is one of the most common genetic causes of childhood fatality. SMA is classified into three groups based on age of onset and achieved motor milestone. Survival Motor Neuron (SMN) gene has been identified as the responsible gene for SMA. From August 2003 until Feb 2007 we have received 93 samples for SMN1 gene deletion analysis from various hospitals in Malaysia. All the patients except for 3 patients were Malaysian (71 Malays, 5 Indians, 9 Chinese and 5 patients are mixed ethnicity). DNA were extracted from blood samples using DNA extraction kit and subjected to SMN/ gene deletion analysis by PCR-RE. Forty nine out of 93 samples (20 type I, 21 type II, and 8 type III) were found to have homozygous deletion of at least exon 7 of the SMN1 gene. Twelve patients (7 type I, 4 type II, 1 type III) showed the presence of the SMN1 gene and the rest were excluded as they did not fulfill the criteria of International SMA Consortium. Deletion analysis of exon 7 of the SMN gene can be an alternative to the existing diagnostic modalities of SMA.
Background: Kawasaki Disease (KD) and acute rheumatic fever are the two leading causes of acquired heart disease in children in the developing countries. Objectives: To determine the epidemiology of KD and its short-term outcome in Malaysian children. Materials & Methods: A retrospective study of patients with a diagnosis of KD at the Kuala Lumpur Hospital from January 1999 to December 2003. Results: 84 patients with KD were seen over the 5 year period. Of these, 52 (61.90%) were male and 32 (38.10%) female. Malays comprised 51 (60.71%), Chinese 30 (35.71%) and Indian 3 (3.57%). Their ages ranged from 2 months to 11 years 1 month old. There were 25 (29.76%) patients less than one year old, 50 (59.52%) aged 1-4 years, 4 (4.76%) aged 5-7 years and 5 (5.95%) were more than 7 years old. Echocardiographic examination during the acute phase showed that 24 patients (28.57%) had coronary artery dilatation; 23 had mild dilatation and one had giant aneurysm involving both coronary arteries. Echocardiographic examination at 8 weeks showed that 5 (21.74%) of the 23 patients with mildly dilated coronary arteries had resolved; the bilateral giant coronary aneurysms remained the same. There was no death due to KD over the 5 year period. Conclusion: KD occurred most commonly in children aged 1-4 years old with a peak at 17 months. There was male preponderance with a male to female ratio of 1.6:1. Despite immunoglobulin therapy, 29% of patients had coronary artery involvementduring the acute phase; 22% of those with mild coronary artery dilatation resolved at 8 weeks after disease onset.
Respiratory papillomatosis is a disease of viral origin which is characterized by warty exophytic lesions in the aerodigestive tract. It is the most common benign lesion of larynx and the second most common cause of hoarseness in children. It has the tendency to recur and to spread through out the entire length of the aerodigestive tract. Although a benign disease, it has the potential of morbid consequences in view of airway complications and the risk of malignant transformation. We report a case of juvenile onset of respiratory papillomatosis and its therapeutic challenges.
We report a rare case of a newborn baby girl who was delivered at 36 weeks of gestation with birth weight of 2680 grams and has ankyloblepharon filiforme adnatum with cleft lip and palate diagnosed at birth. The baby was managed with simple procedures of eyelid bands separation. We also reviewed the embryology of eyelid and its relation with ankyloblepharon formation and discussed the various syndromes and abnormality which may be associated with this eyelid abnormality. The four types of ankyloblepharon which was described by Rosenman and colleague is presented.
Introduction: There are many factors that determine the survival of patients with VSD. Among these include size of VSD, position, pulmonary hypertension, bacterial sepsis, valvular involvement, associated anomalies with VSD, associated syndromes and age at first diagnosis. There has been no published local data as far as we know and this information will be useful especially for consultation with parents. Even though VSD in general has a good prognosis, whenever they have added risks for example pulmonary hypertension then they are at risk of further morbidity and mortality. Objective: To determine the factors that are associated with survival of patients with VSD. Design: Retrospective cohort. Materials and methods: All cases of isolated VSDs admitted to HUSM from 1996 to 2003 were reviewed. Results: Univariate Cox regression of survival time of patients with VSDs revealed that 4 factors had prognostic significance namely bacterial sepsis (HR= 287.7, 95% CI 51.1, 1618.5, P < 0.001), Down syndrome (HR = 14.89, 95% CI 3.00, 73.92, P = 0.001), pulmonary hypertension (HR=14.58, 95% CI 1.69, 125.7, P=0.015) and large VSDs (HR=8.23, 95% CI 1.5, 45, P=0.015). Bacterial sepsis was the only significant prognostic factors for the survival of patients with VSDs using the multivariate Cox proportional hazard model. Conclusion: Bacterial sepsis, pulmonary hypertension, large VSD and Down syndrome were the significant prognostic factors from Univariate Cox analysis, however bacterial sepsis was the only significant prognostic factor from Multivariate Cox analysis.
OBJECTIVE: To assess a better strategy to implement oral iron supplementation in preschool Orang Asli children with high prevalence of iron deficiency, as opposed to the current practice, yet inefficient, of daily oral iron supplementation regime.
METHODS: A randomized controlled trial was conducted in preschool children presenting to a remote health center (Klinik Desa Kenang, Sungai Siput, Perak) with iron deficiency state. Oral iron prescribed as a daily unsupervised dose (group A) was compared to a weekly supervised administration (group B) over eight weeks.
RESULTS: Before intervention, iron deficiency was prevalent in these children (91.2%). The mean baseline haemoglobin and ferritin levels of group A were 9.9 (+/- 1.1) g/dL and 8.9 (+/- 1.3) mg/L respectively, and that of group B were 9.9 (+/-1.2) g/dL and 9.7 (+/- 1.9) mg/L respectively. After eight weeks of treatment, the mean rise in haemoglobin and ferritin levels of group A were 1.2 (+/- 0.6) g/dL and 18.1 (+/- 15.1) mg/ L respectively, as compared to group B, where the mean rise in haemoglobin and ferritin levels were 1.8 (+/- 0.7) g/dL and 35.2 (+/- 21.8) mg/ L respectively. The differences in the rise of haemoglobin and ferritin levels of the two groups were statistically significant (p<0.025). Both regimes were however effective in improving the iron status in a short term (88% in group A and 100% in group B), but group B had a better iron improvement (35.2 +/- 21.8 versus 18.1 +/-15.1 mg/L).
CONCLUSION: It was concluded that the supervised weekly oral iron supplementation regime was more effective than the unsupervised daily supplementation for treating iron deficiency in preschool Orang Asli children. Since iron deficiency is so common in these children and in view of the possibility of poor compliance with the unsupervised regime, an intermittent supervised treatment is proposed as the most effective strategy to address this nutritional problem.